Pediatric Cardiology and Cardiac Surgery
Vol.25 No.6 2009 (800-807)

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Noboru Inamura,1) Futoshi Kayatani,1) Tomoko Kita,1) Yukiko Kawazu,1) Hiroaki Kawata,2) Shigemitsu Iwai,2) and Hidefumi Kishimoto2)

Departments of 1)Pediatric Cardiology and 2)Cardiovascular Surgery, Osaka Medical Center and Research Institute for Maternal and Child Health, Izumi, Osaka, Japan


Background: The configuration of the pulmonary vein (PV) is one of the factors determining the prognosis of patients with asplenia and polysplenia syndrome (APS). However, the configuration of PV in APS when the PV is connected directly to the atrium has not yet been clarified.
Objective: To evaluate the anatomical features of PV connected to the atrium and its influence on clinical outcomes.
Methods: We performed helical CT in 50 patients with APS. Twelve patients had polysplenia and 38 had asplenia syndrome. We evaluated the anatomical features of PV.
Results: In 27 patients, the orifice of a bilateral PV was divided into spinal right and left, and the middle point of the bilateral lower PV orifices as located at the front of the vertebra (central type), and in the other 23 patients, the same point of PV orifices was distant from the vertebra (distant type). PV stenosis (PVS) was found in 12 patients with the central type (5, bilateral PVS; 7, unilateral PVS) and in 12 patients with the distant type (all unilateral PVS). The cause of bilateral PVS in the central type was mainly narrowing of the junction with the atrium. However, the unilateral PV that overrode the vertebra often became stenotic owing to compression by the vertebra, descending aorta, and enlarged atrium. Only one of 5 patients with bilateral PVS survived. Of 19 patients with unilateral PVS, 9 survived. Seven of the 9 patients underwent Fontan procedure. Of the other 26 patients without PVS, 22 survived and 15 underwent the Fontan procedure.
Conclusion: Compressed PVS may play a role in PV obstruction and may be an overlooked risk factor for Fontan candidates with APS.